{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T02:01:41Z","timestamp":1793325701390,"version":"4.5.0"},"reference-count":23,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CJASN"],"published-print":{"date-parts":[[2019,6]]},"abstract":"\n Background and objectives<\/jats:title>\n To evaluate the growth pattern of kidney cyst number and cyst volume in association with kidney size, demographics, and genotypes in autosomal dominant polycystic kidney disease.<\/jats:p>\n <\/jats:sec>\n \n Design, setting, participants, & measurements<\/jats:title>\n \n Kidney cyst number and cyst volume were measured from serial magnetic resonance images, giving a maximum follow-up of 14.23 years, from 241 patients with autosomal dominant polycystic kidney disease (15\u201346 years old at baseline). The growth pattern was analyzed, in association with sex, age, height-adjusted total kidney volume, and genotype, using linear mixed models of repeated measurements and tests of interactions with age (as a time-dependent covariate) to assess rates of change over time. Models were also fit using Irazabal class. Genotypic groups were characterized as either (\n 1<\/jats:italic>\n )\n PKD1<\/jats:italic>\n truncating,\n PKD1<\/jats:italic>\n nontruncating, and\n PKD2<\/jats:italic>\n plus patients with no mutation detected; or (\n 2<\/jats:italic>\n ) in combination with\n PKD1<\/jats:italic>\n mutation strength groups.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Imaging and genetic data were collected (at least one visit) for 236 participants. The mean height-adjusted total cyst number increased exponentially over time from a baseline value of 762 to 1715 at the last clinic visit, while the mean height-adjusted total cyst volume increased exponentially from 305 to 770 ml. Height-adjusted total kidney volume, height-adjusted total cyst number, and height-adjusted total cyst volume were all highly correlated over time. Female participants and participants with larger height-adjusted total kidney volume at baseline showed smaller rates of change in the log of height-adjusted total cyst number and cyst volume.\n PKD1<\/jats:italic>\n was associated with significant increases in both cyst number and volume at a given age, but genotype did not significantly affect the rate of growth.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Both height-adjusted total cyst number and height-adjusted total cyst volume increased exponentially and more than doubled over 14.23 years of follow-up. Compared with\n PKD2<\/jats:italic>\n plus no mutation detected,\n PKD1<\/jats:italic>\n was associated with a greater cyst number and volume at a given age, but no significant difference in the rate of growth.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.2215\/cjn.10360818","type":"journal-article","created":{"date-parts":[[2019,5,14]],"date-time":"2019-05-14T13:35:51Z","timestamp":1557840951000},"page":"823-833","source":"Crossref","is-referenced-by-count":35,"title":["Growth Pattern of Kidney Cyst Number and Volume in Autosomal Dominant Polycystic Kidney Disease"],"prefix":"10.2215","volume":"14","author":[{"given":"Kyongtae T.","family":"Bae","sequence":"first","affiliation":[{"name":"Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Zhou","sequence":"additional","affiliation":[{"name":"Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chengli","family":"Shen","sequence":"additional","affiliation":[{"name":"Biomedical Informatics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas P.","family":"Landsittel","sequence":"additional","affiliation":[{"name":"Biomedical Informatics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhiyuan","family":"Wu","sequence":"additional","affiliation":[{"name":"Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cheng","family":"Tao","sequence":"additional","affiliation":[{"name":"Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arlene B.","family":"Chapman","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of Chicago School of Medicine, Chicago, Illinois;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicente E.","family":"Torres","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1776-2533","authenticated-orcid":false,"given":"Alan S.L.","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Kansas University Medical Center, Kansas City, Kansas;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Mrug","sequence":"additional","affiliation":[{"name":"Department of Medicine, The University of Alabama at Birmingham and"},{"name":"Department of Veterans Affairs Medical Center, Birmingham, Alabama; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William M.","family":"Bennett","sequence":"additional","affiliation":[{"name":"Legacy Good Samaritan Hospital, Portland, Oregon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter C.","family":"Harris","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP)","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2019,5,14]]},"reference":[{"key":"B1-20230813","doi-asserted-by":"crossref","first-page":"479","DOI":"10.2215\/CJN.09500911","article-title":"Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease.","volume":"7","author":"Chapman","year":"2012","journal-title":"Clin J Am Soc Nephrol"},{"key":"B2-20230813","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1038\/ng0695-151","article-title":"The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains.","volume":"10","author":"Hughes","year":"1995","journal-title":"Nat Genet"},{"key":"B3-20230813","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1126\/science.272.5266.1339","article-title":"PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein.","volume":"272","author":"Mochizuki","year":"1996","journal-title":"Science"},{"key":"B4-20230813","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1016\/0888-7543(95)80020-M","article-title":"Evidence for a third genetic locus for autosomal dominant polycystic kidney disease.","volume":"25","author":"Daoust","year":"1995","journal-title":"Genomics"},{"key":"B5-20230813","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1159\/000421651","article-title":"Genetic heterogeneity of polycystic kidney disease in Europe.","volume":"97","author":"Peters","year":"1992","journal-title":"Contrib Nephrol"},{"key":"B6-20230813","doi-asserted-by":"crossref","first-page":"2142","DOI":"10.1681\/ASN.V7102142","article-title":"Linkage, clinical features, and prognosis of autosomal dominant polycystic kidney disease types 1 and 2.","volume":"7","author":"Torra","year":"1996","journal-title":"J Am Soc Nephrol"},{"key":"B7-20230813","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/S0140-6736(98)03495-3","article-title":"Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group.","volume":"353","author":"Hateboer","year":"1999","journal-title":"Lancet"},{"key":"B8-20230813","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1053\/ajkd.2000.17619","article-title":"Increased prevalence of polycystic kidney disease type 2 among elderly polycystic patients.","volume":"36","author":"Torra","year":"2000","journal-title":"Am J Kidney Dis"},{"key":"B9-20230813","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1111\/j.1523-1755.2005.00203.x","article-title":"Modifier genes play a significant role in the phenotypic expression of PKD1.","volume":"67","author":"Fain","year":"2005","journal-title":"Kidney Int"},{"key":"B10-20230813","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1681\/ASN.2004090758","article-title":"Progressive loss of renal function is an age-dependent heritable trait in type 1 autosomal dominant polycystic kidney disease.","volume":"16","author":"Paterson","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"B11-20230813","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1046\/j.1523-1755.2003.00185.x","article-title":"Renal structure in early autosomal-dominant polycystic kidney disease (ADPKD): The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort.","volume":"64","author":"Chapman","year":"2003","journal-title":"Kidney Int"},{"key":"B12-20230813","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1056\/NEJMoa054341","article-title":"Volume progression in polycystic kidney disease.","volume":"354","author":"Grantham","year":"2006","journal-title":"N Engl J Med"},{"key":"B13-20230813","doi-asserted-by":"crossref","first-page":"3013","DOI":"10.1681\/ASN.2006080835","article-title":"Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease.","volume":"17","author":"Harris","year":"2006","journal-title":"J Am Soc Nephrol"},{"key":"B14-20230813","doi-asserted-by":"crossref","first-page":"64","DOI":"10.2215\/CJN.00080605","article-title":"Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort.","volume":"1","author":"Bae","year":"2006","journal-title":"Clin J Am Soc Nephrol"},{"key":"B15-20230813","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1159\/000355375","article-title":"Novel approach to estimate kidney and cyst volumes using mid-slice magnetic resonance images in polycystic kidney disease.","volume":"38","author":"Bae","year":"2013","journal-title":"Am J Nephrol"},{"key":"B16-20230813","doi-asserted-by":"crossref","first-page":"2872","DOI":"10.1681\/ASN.2015050583","article-title":"Predicted mutation strength of nontruncating PKD1 mutations aids genotype-phenotype correlations in autosomal dominant polycystic kidney disease.","volume":"27","author":"Heyer","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"B17-20230813","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1681\/ASN.2013101138","article-title":"Imaging classification of autosomal dominant polycystic kidney disease: A simple model for selecting patients for clinical trials.","volume":"26","author":"Irazabal","year":"2015","journal-title":"J Am Soc Nephrol"},{"key":"B19-20230813","doi-asserted-by":"crossref","first-page":"889","DOI":"10.2215\/CJN.00550110","article-title":"Evidence of extraordinary growth in the progressive enlargement of renal cysts.","volume":"5","author":"Grantham","year":"2010","journal-title":"Clin J Am Soc Nephrol"},{"key":"B20-20230813","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1681\/ASN.2012070650","article-title":"Type of PKD1 mutation influences renal outcome in ADPKD.","volume":"24","author":"Cornec-Le Gall","year":"2013","journal-title":"J Am Soc Nephrol"},{"key":"B21-20230813","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1681\/ASN.2015010016","article-title":"The PROPKD score: A new algorithm to predict renal survival in autosomal dominant polycystic kidney disease.","volume":"27","author":"Cornec-Le Gall","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"B22-20230813","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1097\/01.ASN.0000061774.90975.25","article-title":"Genotype-renal function correlation in type 2 autosomal dominant polycystic kidney disease.","volume":"14","author":"Magistroni","year":"2003","journal-title":"J Am Soc Nephrol"},{"key":"B23-20230813","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.1097\/01.ASN.0000013300.11876.37","article-title":"The position of the polycystic kidney disease 1 (PKD1) gene mutation correlates with the severity of renal disease.","volume":"13","author":"Rossetti","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"B24-20230813","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.2215\/CJN.00900112","article-title":"Detected renal cysts are tips of the iceberg in adults with ADPKD.","volume":"7","author":"Grantham","year":"2012","journal-title":"Clin J Am Soc Nephrol"}],"container-title":["Clinical Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/01277230-201906000-00009","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T13:36:56Z","timestamp":1786369016000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/01277230-201906000-00009"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,5,14]]},"references-count":23,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2019]]}},"URL":"https:\/\/doi.org\/10.2215\/cjn.10360818","relation":{},"ISSN":["1555-9041","1555-905X"],"issn-type":[{"value":"1555-9041","type":"print"},{"value":"1555-905X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,5,14]]}}}